Morphologic Aspects of Interstitial Pneumonia With Autoimmune Features.
Morphologic Aspects of Interstitial Pneumonia With Autoimmune Features.
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DOI：
10.5858/arpa.2017-0528-ra
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发表时间：
2018-08
影响因子：
4.6
通讯作者：
E. Nascimento;B. Baldi;M. Sawamura;M. Dolhnikoff
中科院分区：
文献类型：
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作者：
E. Nascimento;B. Baldi;M. Sawamura;M. Dolhnikoff
背景-间质性肺病是几种结缔组织疾病中常见的并发症，可导致显著的发病率和死亡率。与结缔组织病患者相似，临床和血清学特征提示自身免疫，但没有确诊的特定结缔组织病的患者中，有一大部分患者表现为相关的间质性肺疾病。这些患者使用了不同的有争议的命名法，如未分化结缔组织病相关的间质性肺疾病、肺显性结缔组织病和自身免疫性间质性肺疾病。由于需要更好地了解和标准化具有自身免疫特征的间质性肺病这一实体，以及需要为患者制定适当的管理方案，因此在2015年引入了一个新术语：具有自身免疫特征的间质性肺炎。这一新的分类需要更好地理解其诊断影响和其形态方面对患者预后的影响。目的：回顾具有自身免疫性特征的间质性肺炎的诊断标准，并着重讨论其形态学特征。数据来源：这篇综述是基于现有的文献，以及病理学、放射学和临床经验。结论：具有自身免疫特征的间质性肺炎似乎识别出不同预后的不同亚组患者。研究表明，非特异性间质性肺炎和普通型间质性肺炎是最常见的病理类型，对普通型间质性肺炎对生存的影响显示出不同的结果。有必要进行前瞻性研究，以更好地确定这一亚群，并确定这些患者的预后和适当的临床处理。
CONTEXT - Interstitial lung disease, a common complication observed in several connective tissue diseases, causes significant morbidity and mortality. Similar to individuals with connective tissue diseases, a significant subgroup of patients with clinical and serologic characteristics suggestive of autoimmunity but without confirmed specific connective tissue disease presents with associated interstitial lung disease. These patients have been classified using different controversial nomenclatures, such as undifferentiated connective tissue disease-associated interstitial lung disease, lung-dominant connective tissue disease, and autoimmune featured interstitial lung disease. The need for a better understanding and standardization of this entity, interstitial lung disease with autoimmune features, and the need for an adequate management protocol for patients resulted in the introduction of a new terminology in 2015: interstitial pneumonia with autoimmune features. This new classification requires a better comprehension of its diagnostic impact and the influence of its morphologic aspects on the prognosis of patients. OBJECTIVE - To review the diagnostic criteria for interstitial pneumonia with autoimmune features, with an emphasis on morphologic aspects. DATA SOURCES - The review is based on the available literature, and on pathologic, radiologic, and clinical experience. CONCLUSIONS - The interstitial pneumonia with autoimmune features classification seems to identify a distinct subgroup of patients with different prognoses. Studies show that nonspecific interstitial pneumonia and usual interstitial pneumonia are the most prevalent morphologic patterns and show discrepant results on the impact of the usual interstitial pneumonia pattern on survival. Prospective investigations are necessary to better define this subgroup and to determine the prognosis and appropriate clinical management of these patients.